Jean-Christophe Thalabard

Late-Onset Adrenal Hyperplasia in Hirsutism

We studied the incidence of late-onset adrenal hyperplasia as a cause of hirsutism, its association with the major histocompatibility complex, and its clinical expression. Twenty-four of 400 women seen because of hirsutism were found to have late-onset adrenal hyperplasia, diagnosed on the basis of a high plasma level of 17-hydroxyprogesterone, and its marked increase after ACTH stimulation. The degree of hirsutism varied widely. Plasma antigen levels were high, especially the level of androstenedione, whereas 5 alpha-reductase activity, considered to be a good index of peripheral androgen utilization, showed frequent normal or low values. The 24 patients were genotyped, along with 84 family members, and plasma hormones were measured in the family members. We found a high correlation between late-onset adrenal hyperplasia and HLA antigens B14 and Aw33. Similar biologic profiles were observed in the patients and those of their siblings who were HLA identical (n = 9), confirming that late-onset adrenal hyperplasia is linked to the histocompatibility complex. These nine siblings had no hirsutism. We therefore conclude that the role of skin sensitivity to androgens is important in determining the clinical expression of this disorder.

Fetal lung volume measurement by magnetic resonance imaging in congenital diaphragmatic hernia

Objective To study the potential for prenatal magnetic resonance imaging to predict pulmonary hypoplasia in congenital diaphragmatic hernia.

Three‐dimensional ultrasonographic assessment of fetal lung volume as prognostic factor in isolated congenital diaphragmatic hernia

Objective  To evaluate the potential of three‐dimensional ultrasound to predict outcome in congenital diaphragmatic hernia.

Fetal Lung Volume Estimated by 3-Dimensional Ultrasonography and Magnetic Resonance Imaging in Cases With Isolated Congenital Diaphragmatic Hernia

Objective. To assess the agreement of 3‐dimensional ultrasonography and magnetic resonance imaging in estimating fetal lung volume in cases with isolated congenital diaphragmatic hernia. Methods. Fetal lung volume was measured in 11 cases of congenital diaphragmatic hernia (10 left and 1 right) by 3‐dimensional ultrasonography and magnetic resonance imaging. These examinations were performed during the same week. The operators were blinded to each others results. Intraclass correlation was used to evaluate the agreement between 3‐dimensional ultrasonography and magnetic resonance imaging estimations of the ipsilateral, contralateral, and total fetal lung volume. A Bland‐Altman graph was plotted to detect possible discordant observations. Results. The global intraclass correlation coefficient between magnetic resonance imaging and 3‐dimensional ultrasonographic measurement of fetal lung volume was 0.94 (95% confidence interval, 0.78–0.98) with no outliers observed on the Bland‐Altman plot. Conclusions. There is a good agreement between 3‐dimensional ultrasonography and magnetic resonance imaging for fetal lung volume estimation in cases with congenital diaphragmatic hernia.

A nomogram of fetal lung volumes estimated by 3-dimensional ultrasonography using the rotational technique (virtual organ computer-aided analysis).

Objective. The purpose of this study was to build a nomogram of normal fetal lung volumes throughout gestational age estimated by 3‐dimensional ultrasonography using the rotational technique (Virtual Organ Computer‐Aided Analysis [VOCAL]; GE Healthcare, Kretztechnik, Zipf, Austria). Methods. Fetal lung volume was assessed in 146 healthy fetuses by 3‐dimensional ultrasonography using the technique of rotation of the multiplanar imaging (VOCAL). Inclusion criteria were healthy women with singleton normal pregnancies, normal fetal morphologic ultrasonographic findings, reliable dating established by dates and by ultrasonographic measurement of the crown‐lump length in the first trimester, and gestational age from 20 to 37 weeks. Exclusion criteria were discordance between clinical and ultrasonographic dating, patients lost to follow‐up, and birth weight disorders. Each patient was scanned once during pregnancy. Results. The right, left, and total mean pulmonary volumes ranged, respectively, from 5.37, 4.66, and 9.95 cm3 at 20 weeks to 46.06, 37.34, and 84.35 cm3 at 37 weeks. The logistic transformation analysis yielded the following formulas: right lung volume = exp(4.07/[1 + exp(21.90 − gestational age/5.44)]); left lung volume = exp(3.82/(1 + exp[22.03 − gestational age/5.17)]); and, total lung volume = exp(4.72/[1 + exp(20.30 − gestational age/6.05)]). Conclusions. A new nomogram of fetal lung (right, left, and total) volumes throughout gestational age using the rotational technique (VOCAL) is described, and reference values have been generated.

Risk factors for breast fibroadenoma in young women

In a case-control study, 178 women diagnosed with fibroadenoma (FA) between 1976 and 1982 were compared with 178 age-matched controls in order to assess the risk factors for FA. Women who had a previous diagnosis of breast disease were excluded. The odds ratios (OR) of FA were calculated for different risk factors of breast disease from bivariate analysis as well as multivariate regression analysis. The highest ORs were found for a previous premenstrual mastalgia before first childbirth and a familial history (FH) of breast cancer (BC). Oral contraceptive (OC) use before a first full-term pregnancy (FFTP) does not modify this risk. Also current use of standard oral contraceptives (SOC) containing 50 micrograms of ethinyl estradiol per pill appeared to be protective and the correlation with FA was negative. These findings underline: (a) the predictive value of premenstrual mastalgia for the subsequent development of breast disease; (b) only current high-dose OC use is protective.

The use of the antiprogestin RU486 (Mifepristone): As an abortifacient in early pregnancy — clinical and pathological findings; predictive factors for efficacy☆

RU486, a potent antiprogesterone steroid was administered to 124 women requesting therapeutic abortion. All were less than 49 days from their last menstrual period. Ten of these subjects (Group I) received high doses of RU486 in a decremental dose regimen (400, 300, 200 and 100 mg/day) over 4 successive days and 14 received 50 mg/day for 7 days (Group II). A further 50 subjects (Group III) received 100 mg/day for seven days and the remaining 50 subjects (Group IV) received 450 mg in a single dose. In the first three groups, half the daily dose was given in the morning and the remainder in the evening. Blood was collected before, and on Days 4 and 7 and then once a week after commencing therapy until disappearance of circulating beta HCG. In addition to beta HCG, estradiol-17 beta (E2), progesterone (P), cortisol, and various metabolic and hematological parameters were measured. Plasma RU486 concentrations were also assayed in Group II, III and IV subjects on Day 7 of therapy and in some cases on Days 14 and 21. Ultrasonography was performed in all cases on Day 1 and on Day 14. All the patients bled within five days following RU486 administration, for 1 to 21 days. A complete abortion occurred in 60% in Group I, 50% in Group II, 86% in Group III, and 80% in Group IV. The difference between the last two groups and the first two was significant at p less than 0.01. The non-responders were submitted to a uterine vacuum aspiration. A stepwise discriminant analysis was performed and indicated that the best predictors of the outcome of therapy were beta HCG values and the gestational sac diameter. With these criteria, the prediction was accurate in 86.4% of the cases. The best results were obtained in the cases where the ultrasonic measurement of gestational sac was under 10 mm in diameter and the initial beta HCG values under 15,000 mIU/ml. Among the observed side effects were moderate pelvic cramps (20.9%), nausea (27%), fainting (4.8%); 61.3% of the women complained of fatigue. Heavy bleeding occurred in 15.3% of the women but only one of them required blood transfusion. In the patients with complete abortion, beta HCG values decreased to below 500 mIU/ml by Day 14 (but in 11 cases values fell below 2,000 mIU/ml only by Day 21). Plasma estradiol and progesterone also fell. Cortisol levels increased during therapy especially in subjects of Group I, but returned to basal values after termination of treatment.(ABSTRACT TRUNCATED AT 400 WORDS)

OC169: Prediction of pulmonary hypoplasia with MRI

Prediction of pulmonary hypoplasia may be relevant in several conditions associated with impaired pulmonary growth, particularly in chronic severe oligohydramnios and congenital diaphragmatic hernia (CDH). Most experience has been gathered with CDH. The post-natal clinical evolution of fetuses with this condition is essentially associated with the degree of pulmonary hypoplasia, which will eventually determine the degree of pulmonary insufficiency and pulmonary hypertension. The development of strategies for in utero treatment of CDH prompted the search of prognostic signs predicting lethal pulmonary hypoplasia. The best predictor of pulmonary hypoplasia in CDH seems to be the size of the contralateral lung. Lung-to-head ratio (LHR) is the validated used parameter. LHR is calculated in a four chamber view, as the area of the right lung (anterior by transversal diameters) divided by the head circumference. The prognostic accuracy of LHR has been evaluated in left CDH cases. According to different authors, fetuses with LHR values below 1.0 have a high risk for severe pulmonary hypoplasia and neonatal death. LHR is still limited in predicting accurately the degree of pulmonary hypoplasia. Calculation of the right lung diameters may be subject to interobserver variability. It is expected that calculation of right lung volumetry, either by 3D ultrasound or MRI, could allow more precise estimations of the degree of pulmonary hypoplasia. While estimating the lung size or volume is probably the best predictor we have, it is likely that other factors, such as the time of onset in early pregnancy, may determine variable degrees of hypoplasia which may explain the variability in the survival of apparently similar cases. The use of Doppler indices in the fetal pulmonary artery has also been proposed to evaluate the degree of pulmonary hypoplasia. Results to date have showed a marked lack of accuracy both in oligohydramnios as in CDH, but according to recent studies it could constitute a valuable tool when used in combination with other clinical and/or biometrical parameters.

P09.11: What is the precision of 3DUS in estimating fetal lung volume in congenital diaphragmatic hernia?

2); cystic lung disease (n = 2); congenital diaphragmatic hernia (n = 3); heterotaxia with right sided stomach (n = 2), double bubble and bowel obstructions (n = 2) cardiac defects (n = 8) renal malformations N = 4. Conclusions: Malformations affecting the fluid filled organs can be easily demonstrated with the transparent minimum mode. This method is also easy in quickly demonstrating normality of organs as stomach position and filling.